Anorectal Malformations Complications
Perineal fistulas in males are commonly missed in the newborn period because the baby passes meconium through the small fistulous opening, and no one notices the abnormal anal anatomy. These patients typically present in the first year of life with severe constipation.
By the time a missed perineal fistula is diagnosed, the rectum and sigmoid have dilated because stool has been passing through a very tiny fistulous orifice which is not normal anal or rectal mucosa.
The current standard is to check temperature on the forehead or in the ear rather than rectally, so if you don't look at the anus, you might not know there is a malformation.
Relocating a perineal fistula into the sphincters does not completely fix the constipation, though it improves the anatomy by making the hole adequately sized and lined by mucosa.
Patients with uncorrected perineal fistulas can have some semblance of continence with formed stool, but with loose stool or athletic activity they will soil because they cannot completely close the anteriorly located hole when squeezing their sphincters.
A newborn anus should accept a size 12 Hagar dilator, and a one-year-old should accept a size 15.
A bucket handle (a lifted skin tag that you can pass a probe underneath) is consistent with a perineal fistula even if you cannot see the fistula itself.
Perineal fistula in females is probably the most confounding diagnosis in pediatric colorectal surgery, with many patients either being missed or overdiagnosed.
Expert opinionMarc Levitt7:28
Diagnostic criteria for perineal fistula in females: inadequate perineal body (hole too close to vagina), inadequate hole size, and hole not centered in the sphincter.
If the anal opening in a female is adequate size and centered in the sphincter, even if it appears slightly anterior with a short perineal body, that patient does not need surgery. The perineal body will lengthen with growth.
An anesthesia nerve stimulator ($150) with appropriate needle probes works as well as a dedicated Pena stimulator ($15,000) for intraoperative sphincter mapping.
The vast majority of male ARM patients have a rectourethral fistula. It is important not to approach these primarily because you don't know where the rectum is—it could be at bladder neck, prostatic, or bulbar level.
If you open posterior sagittal looking for a rectourethral fistula without knowing the location, you will find something midline, white, and shiny that might be the urinary tract, not the rectum.
Cloacas can be missed in the newborn period. Dr. Levitt saw a six-month-old who presented with constipation and was found to have an undiagnosed cloaca with no hint of an anal opening.
Ambiguous genitalia (clitoromegaly from endocrine stimulation) presents with a urogenital sinus but a completely normal anus. This is different from a cloaca, which has no anus and no endocrine problem.
The most common colostomy error is opening too distal in the sigmoid, which restricts the ultimate pull-through by the location of the colostomy or mucous fistula.
Incompletely diverting loop colostomies allow stool to spill across and contaminate the distal segment, leading to urinary tract infections.
Transverse colostomies are problematic because they can prolapse, and if there is a large rectourethral fistula, the left colon absorbs all the urine (which doesn't come out the mucous fistula), causing acidosis.
Dr. Levitt's preference is a very proximal sigmoid colostomy with separated stomas, leaving the entire sigmoid loop for the pull-through. He makes the mucous fistula very tiny and flat.
Prolapse is related to where in the colon you choose to do the colostomy. Mid-transverse: both sides can prolapse. Hepatic flexure: only distal can prolapse. Proximal sigmoid: only distal (mucous fistula) can prolapse because left colon is fixed to retroperitoneum.
Dr. Levitt marks the anoplasty location by drawing a circle around the pinkish ellipse where it stimulates on the skin surface BEFORE making the incision, to avoid getting lost when looking at jumping muscles from the stimulator.
Really good surgeons have put anuses in crazy places because they don't have a sense of what's the center once everything is disrupted and open.
The distal colostogram is an absolutely vital study. Many mistakes are made because of a poorly done study and misinterpretation.
The basic questions the distal colostogram must answer: Where is the rectum? How low is it? Is it reachable posterior sagittally or better approached laparoscopically? What is its relationship to the urinary tract?
The common colostogram mistake is not giving enough contrast and pressure into the distal segment, giving a false impression that the rectum is high or that there is no fistula.
If you see a straight line flattening of the rectum corresponding to the pubococcygeal line, the radiologist did not give enough contrast or pressure. You need to overcome the PC line (the sphincters compressing the distal rectum) to see the bulging rectum and fistula.
Fistula classification: if the fistula is at the urethral 'elbow' or below, it's bulbar. Above the elbow is prostatic. At the bladder neck is bladder neck fistula.
If the rectum is bulbous, it might be reachable posterior sagittally and hard to do laparoscopically because of the girth. If it's tapered, you're better off laparoscopically.
If you don't know where the rectum is and open posterior sagittal, you will find a whitish, shiny structure and may think it's the rectum. Often it's the bladder neck.
You avoid bladder neck injury by knowing exactly where the rectum is from a properly done distal colostogram. When you open posterior sagittal, you know the rectum is right under the coccyx (prostatic) or distal to the coccyx (bulbar), or it isn't posterior sagittal at all (bladder neck—do laparoscopy).